Table 1.
Patient and tumor characteristics for 21 patients with neurofibromatosis 1 and a diagnosis of malignant peripheral nerve-sheath tumors between 1993 and 2003
| Age, years | |
|---|---|
| Median (range) |
31 (18–79) |
| Tumor size, cm |
|
| Median (range) |
13 (3–28) |
| Gender, n (%) |
|
| Male |
12 (57) |
| Female |
9 (43) |
| Metastases at diagnosis, n (%) |
|
| Absent |
18 (86) |
| Present |
3 (14) |
| Type of surgery, n (%) |
|
| R0, complete resection |
1 (5) |
| R1, incomplete resection (microscopically) |
3 (14) |
| R2, incomplete resection (macroscopically) |
7 (33) |
| No surgery |
10 (48) |
| Histological grade (FNCLCC), n (%) |
|
| 1 |
1 (5) |
| 2 |
8 (38) |
| 3 |
12 (57) |
| Pathological staging, n (%) |
|
| T1 |
3 (14) |
| T2 |
18 (86) |
| Tumor site, n (%) |
|
| Head and neck |
3 (14) |
| Trunk |
4 (19) |
| Extremity |
8 (38) |
| Abdomen or pelvis |
6 (29) |
| Chemotherapy, n (%) |
|
| Single agent |
6 (29) |
| Combination |
15 (71) |
| Type of chemotherapy, n (%) |
|
| Doxorubicin |
5 (24) |
| Ifosfamide |
1 (5) |
| Ifosfamide + anthracycline |
11 (52) |
| Ifosfamide + anthracycline + platinum salt | 4 (19) |
Abbreviations: FNCLCC Federation Nationale des Centres de Lutte Contre le Cancer; T tumor.